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Anti-MuSK-positive myasthenia gravis patient antibody causes aberrant activation of MuSK and disassembly of acetylcholine receptor clusters

Nazanin Ghazanfari, Othon Gervásio, Shyuan Thieu Ngo, Stephen Reddel, William Donald Phillips

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Abstract

Purpose: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease of the neuromuscular junction (NMJ) that causes impaired neuromuscular transmission and muscle weakness. Muscle Specific Kinase (MuSK) is recognized as the auto-antigen in about 10% of MG patients. Activation of MuSK by neural agrin is essential for acetylcholine receptor (AChR) clustering at the NMJ. Here we aimed to define the mechanisms by which anti-MuSK antibodies interfere with the postsynaptic AChR cluster.Conclusion: Anti-MuSK auto-antibodies cross-link and activate MuSK. This somehow causes the disassembly of large pre-existing AChR clusters.

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What this paper is about

Purpose: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease of the neuromuscular junction (NMJ) that causes impaired neuromuscular transmission and muscle weakness. Muscle Specific Kinase (MuSK) is recognized as the auto-antigen in about 10% of MG patients. Activation of MuSK by neural agrin is essential for acetylcholine receptor (AChR) clustering at the NMJ. Here we aimed to define the mechanisms by which anti-MuSK antibodies interfere with the postsynaptic AChR cluster.Conclusion: Anti-MuSK auto-antibodies cross-link and activate MuSK. This somehow causes the disassembly of large pre-existing AChR clusters.

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Available abstract

Purpose: Myasthenia gravis (MG) is an antibody-mediated autoimmune disease of the neuromuscular junction (NMJ) that causes impaired neuromuscular transmission and muscle weakness. Muscle Specific Kinase (MuSK) is recognized as the auto-antigen in about 10% of MG patients. Activation of MuSK by neural agrin is essential for acetylcholine receptor (AChR) clustering at the NMJ. Here we aimed to define the mechanisms by which anti-MuSK antibodies interfere with the postsynaptic AChR cluster.Conclusion: Anti-MuSK auto-antibodies cross-link and activate MuSK. This somehow causes the disassembly of large pre-existing AChR clusters.

Key concepts: Myasthenia gravis, Agrin, Acetylcholine receptor, Neuromuscular junction, Neuromuscular transmission, Postsynaptic potential, Antibody, Acetylcholine

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Anti-MuSK-positive myasthenia gravis patient antibody causes aberrant activation of MuSK and disassembly of acetylcholine receptor clusters — Research Paper | ScholarLens